Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study

Aleksander Kempny; Cristel Sørensen Hjortshøj; Hong Gu; Wei Li; Alexander R Opotowsky; Michael J Landzberg; Annette Schophuus Jensen; Lars Søndergaard; Mette-Elise Estensen; Ulf Thilén; Werner Budts; Barbara J Mulder; Ilja Blok; Lidia Tomkiewicz-Pająk; Kamil Szostek; Michele D'Alto; Giancarlo Scognamiglio; Katja Prokšelj; Gerhard-Paul Diller; Konstantinos Dimopoulos; Stephen J Wort; Michael A Gatzoulis

doi:10.1161/CIRCULATIONAHA.116.023033

Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study

Aleksander Kempny, Cristel Sørensen Hjortshøj, Hong Gu, Wei Li, Alexander R Opotowsky, Michael J Landzberg, Annette Schophuus Jensen, Lars Søndergaard, Mette-Elise Estensen, Ulf Thilén, Werner Budts, Barbara J Mulder, Ilja Blok, Lidia Tomkiewicz-Pająk, Kamil Szostek, Michele D'Alto, Giancarlo Scognamiglio, Katja Prokšelj, Gerhard-Paul Diller, Konstantinos DimopoulosStephen J Wort, Michael A Gatzoulis

Institut for Klinisk Medicin

54 Citationer (Scopus)

Abstract

Background: Eisenmenger syndrome is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of Eisenmenger syndrome patients. Methods: In a multicenter approach, we identified adults with Eisenmenger syndrome under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic, and laboratory parameters. Results: We studied 1098 patients (median age, 34.4 years; range, 16.1-84.4 years; 65.1% female; 31.9% with Down syndrome). The majority had a posttricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pretricuspid lesion (n=140, 12.7%). Over a median follow-up of 3.1 years (interquartile range, 1.4-5.9), allowing for 4361.6 patient-years observation, 278 patients died and 6 underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41/10 years; 95% confidence interval [CI], 1.24-1.59; P<0.001), pretricuspid shunt (HR, 1.56; 95% CI, 1.02-2.39; P=0.041), oxygen saturation at rest (HR, 0.53/10%; 95% CI, 0.43-0.65; P<0.001), presence of sinus rhythm (HR, 0.53; 95% CI, 0.32-0.88; P=0.013), and presence of pericardial effusion (HR, 2.41; 95% CI, 1.59-3.66; P<0.001) remained significant predictors of death. Conclusions: There is significant premature mortality among contemporary adults with Eisenmenger syndrome. We report, herewith, a multivariable mortality risk stratification model based on 5 simple, noninvasive predictors of death in this population.

Originalsprog	Engelsk
Tidsskrift	Circulation
Vol/bind	135
Udgave nummer	15
Sider (fra-til)	1432-1440
ISSN	0009-7322
DOI	https://doi.org/10.1161/CIRCULATIONAHA.116.023033
Status	Udgivet - 11 apr. 2017

FN’s Verdensmål

Dette resultat bidrager til følgende verdensmål

Adgang til dokumentet

10.1161/CIRCULATIONAHA.116.023033

http://hdl.handle.net/10044/1/44479Licens: CC BY-NC-ND

Citationsformater

Kempny, A., Hjortshøj, C. S., Gu, H., Li, W., Opotowsky, A. R., Landzberg, M. J., Jensen, A. S., Søndergaard, L., Estensen, M-E., Thilén, U., Budts, W., Mulder, B. J., Blok, I., Tomkiewicz-Pająk, L., Szostek, K., D'Alto, M., Scognamiglio, G., Prokšelj, K., Diller, G-P., ... Gatzoulis, M. A. (2017). Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study. Circulation, 135(15), 1432-1440. https://doi.org/10.1161/CIRCULATIONAHA.116.023033

Kempny, A, Hjortshøj, CS, Gu, H, Li, W, Opotowsky, AR, Landzberg, MJ, Jensen, AS, Søndergaard, L, Estensen, M-E, Thilén, U, Budts, W, Mulder, BJ, Blok, I, Tomkiewicz-Pająk, L, Szostek, K, D'Alto, M, Scognamiglio, G, Prokšelj, K, Diller, G-P, Dimopoulos, K, Wort, SJ & Gatzoulis, MA 2017, 'Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study', Circulation, bind 135, nr. 15, s. 1432-1440. https://doi.org/10.1161/CIRCULATIONAHA.116.023033

@article{dde30a332fb44c7aafbdfb192da8f971,

title = "Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study",

abstract = "Background: Eisenmenger syndrome is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of Eisenmenger syndrome patients. Methods: In a multicenter approach, we identified adults with Eisenmenger syndrome under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic, and laboratory parameters. Results: We studied 1098 patients (median age, 34.4 years; range, 16.1-84.4 years; 65.1% female; 31.9% with Down syndrome). The majority had a posttricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pretricuspid lesion (n=140, 12.7%). Over a median follow-up of 3.1 years (interquartile range, 1.4-5.9), allowing for 4361.6 patient-years observation, 278 patients died and 6 underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41/10 years; 95% confidence interval [CI], 1.24-1.59; P<0.001), pretricuspid shunt (HR, 1.56; 95% CI, 1.02-2.39; P=0.041), oxygen saturation at rest (HR, 0.53/10%; 95% CI, 0.43-0.65; P<0.001), presence of sinus rhythm (HR, 0.53; 95% CI, 0.32-0.88; P=0.013), and presence of pericardial effusion (HR, 2.41; 95% CI, 1.59-3.66; P<0.001) remained significant predictors of death. Conclusions: There is significant premature mortality among contemporary adults with Eisenmenger syndrome. We report, herewith, a multivariable mortality risk stratification model based on 5 simple, noninvasive predictors of death in this population.",

keywords = "Adolescent, Adult, Aged, Aged, 80 and over, Biomarkers, Echocardiography, Eisenmenger Complex/diagnosis, Electrocardiography, Female, Follow-Up Studies, Humans, Male, Middle Aged, Oxygen Consumption, Phenotype, Prognosis, Proportional Hazards Models, Risk Assessment, Risk Factors, Severity of Illness Index, Walk Test, Young Adult",

author = "Aleksander Kempny and Hjortsh{\o}j, {Cristel S{\o}rensen} and Hong Gu and Wei Li and Opotowsky, {Alexander R} and Landzberg, {Michael J} and Jensen, {Annette Schophuus} and Lars S{\o}ndergaard and Mette-Elise Estensen and Ulf Thil{\'e}n and Werner Budts and Mulder, {Barbara J} and Ilja Blok and Lidia Tomkiewicz-Paj{\c a}k and Kamil Szostek and Michele D'Alto and Giancarlo Scognamiglio and Katja Prok{\v s}elj and Gerhard-Paul Diller and Konstantinos Dimopoulos and Wort, {Stephen J} and Gatzoulis, {Michael A}",

note = "{\textcopyright} 2016 American Heart Association, Inc.",

year = "2017",

month = apr,

day = "11",

doi = "10.1161/CIRCULATIONAHA.116.023033",

language = "English",

volume = "135",

pages = "1432--1440",

journal = "Circulation",

issn = "0009-7322",

publisher = "AHA/ASA",

number = "15",

}

TY - JOUR

T1 - Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome

T2 - A Multicenter Study

AU - Kempny, Aleksander

AU - Hjortshøj, Cristel Sørensen

AU - Gu, Hong

AU - Li, Wei

AU - Opotowsky, Alexander R

AU - Landzberg, Michael J

AU - Jensen, Annette Schophuus

AU - Søndergaard, Lars

AU - Estensen, Mette-Elise

AU - Thilén, Ulf

AU - Budts, Werner

AU - Mulder, Barbara J

AU - Blok, Ilja

AU - Tomkiewicz-Pająk, Lidia

AU - Szostek, Kamil

AU - D'Alto, Michele

AU - Scognamiglio, Giancarlo

AU - Prokšelj, Katja

AU - Diller, Gerhard-Paul

AU - Dimopoulos, Konstantinos

AU - Wort, Stephen J

AU - Gatzoulis, Michael A

PY - 2017/4/11

Y1 - 2017/4/11

N2 - Background: Eisenmenger syndrome is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of Eisenmenger syndrome patients. Methods: In a multicenter approach, we identified adults with Eisenmenger syndrome under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic, and laboratory parameters. Results: We studied 1098 patients (median age, 34.4 years; range, 16.1-84.4 years; 65.1% female; 31.9% with Down syndrome). The majority had a posttricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pretricuspid lesion (n=140, 12.7%). Over a median follow-up of 3.1 years (interquartile range, 1.4-5.9), allowing for 4361.6 patient-years observation, 278 patients died and 6 underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41/10 years; 95% confidence interval [CI], 1.24-1.59; P<0.001), pretricuspid shunt (HR, 1.56; 95% CI, 1.02-2.39; P=0.041), oxygen saturation at rest (HR, 0.53/10%; 95% CI, 0.43-0.65; P<0.001), presence of sinus rhythm (HR, 0.53; 95% CI, 0.32-0.88; P=0.013), and presence of pericardial effusion (HR, 2.41; 95% CI, 1.59-3.66; P<0.001) remained significant predictors of death. Conclusions: There is significant premature mortality among contemporary adults with Eisenmenger syndrome. We report, herewith, a multivariable mortality risk stratification model based on 5 simple, noninvasive predictors of death in this population.

AB - Background: Eisenmenger syndrome is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of Eisenmenger syndrome patients. Methods: In a multicenter approach, we identified adults with Eisenmenger syndrome under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic, and laboratory parameters. Results: We studied 1098 patients (median age, 34.4 years; range, 16.1-84.4 years; 65.1% female; 31.9% with Down syndrome). The majority had a posttricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pretricuspid lesion (n=140, 12.7%). Over a median follow-up of 3.1 years (interquartile range, 1.4-5.9), allowing for 4361.6 patient-years observation, 278 patients died and 6 underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis, only age (hazard ratio [HR], 1.41/10 years; 95% confidence interval [CI], 1.24-1.59; P<0.001), pretricuspid shunt (HR, 1.56; 95% CI, 1.02-2.39; P=0.041), oxygen saturation at rest (HR, 0.53/10%; 95% CI, 0.43-0.65; P<0.001), presence of sinus rhythm (HR, 0.53; 95% CI, 0.32-0.88; P=0.013), and presence of pericardial effusion (HR, 2.41; 95% CI, 1.59-3.66; P<0.001) remained significant predictors of death. Conclusions: There is significant premature mortality among contemporary adults with Eisenmenger syndrome. We report, herewith, a multivariable mortality risk stratification model based on 5 simple, noninvasive predictors of death in this population.

KW - Adolescent

KW - Adult

KW - Aged

KW - Aged, 80 and over

KW - Biomarkers

KW - Echocardiography

KW - Eisenmenger Complex/diagnosis

KW - Electrocardiography

KW - Female

KW - Follow-Up Studies

KW - Humans

KW - Male

KW - Middle Aged

KW - Oxygen Consumption

KW - Phenotype

KW - Prognosis

KW - Proportional Hazards Models

KW - Risk Assessment

KW - Risk Factors

KW - Severity of Illness Index

KW - Walk Test

KW - Young Adult

U2 - 10.1161/CIRCULATIONAHA.116.023033

DO - 10.1161/CIRCULATIONAHA.116.023033

M3 - Journal article

C2 - 27979875

SN - 0009-7322

VL - 135

SP - 1432

EP - 1440

JO - Circulation

JF - Circulation

IS - 15

ER -

Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study

Abstract

FN’s Verdensmål

Adgang til dokumentet

Fingeraftryk

Citationsformater